We came home the day after radiation ended back in January, thinking we would have a few weeks at home, an MRI then right back to Boston. But, as scheduling usually goes, it took 2 months to schedule an MRI (which in the end, he did UNSEDATED!!!! His first ever! It was only a half hour and only the brain, but still, that was huge! Had we known he wouldn't need anesthesia, it probably could've been scheduled much sooner). Then to get results back...another few weeks. In fact, it took a full month to hear back from our team. Here are the results of 'the spot:' UNMH compared it to a different scan, so they concluded no change. MGH, where Emmett did proton radiation, concluded about a 20% shrinkage and clean auditory nerves now. Dr. MacDonald, our radiation oncologist, was pleased with those results she said and strongly recommends a systemic therapy now. DFCI, our main team, reported they see 50% shrinkage and concluded that the spot is very radiosensitive and hope for continued shrinking. Initially, when we saw UNMH's results, we were excited because if it hadn't responded at all to radiation, that strengthens the case that it is NOT cancer. Because of that news, we felt that we would be done with treatment for now and just continue to watch and scan like normal. However, just a few days later, that peace was brought to a quick end with MGH's news. I mean, shrinking for cancer is good news, but 'probably not cancer' is obviously better. But we were very happy that the doctors were seeing good results from the radiation. We are back to considering our next treatment. Dr. Chi is still not as strongly convinced we need further treatment as Dr. MacDonald is.
Here are the options we're considering for those who like details or are reading this blog in search of direction for their own child's treatment. The first is a study from the company Epizyme, who has produced a new drug called EZH2. This is Dr. Chi's research and is not traditional chemo, but a new type of drug that is hopefully the way treatment is going in the future. Instead of defining cancer by where is occurs in the body, they are defining tumors by what they genetically have in common. For ATRT, it is an INI-1 deficient tumor. This gene normally is what codes for tumor suppression, or what recognizes tumors and then tells the body to squash it. So you can imagine if you have a mutation or genetic error in this gene, then your body does not produce the right proteins to send the signals to squash the tumor and becomes very problematic. What this new drug does is hopefully treat this deficiency. (At least this is my basic understanding) They have given the drug to 5 adults and 4 of them had shrinking; 1 of which had the tumor totally gone. We are told this is HUGE success for such an early study. Fast forward maybe 2 years and they are just giving this to children now: 6-12 children worldwide have taken it starting in February. The side effects so far seem to be mostly tiredness. This was funded NOT by any donations to the Jimmy Fund but entirely by a certain team for bike ride fundraiser, headed by an incredibly enthusiastic mom who already lost a child to this disease. Janet Larkin O'Shea and her team, Team Lick Cancer, have raised around $250,000 (yes a QUARTER MILLION DOLLARS!) each year and give it to Dr. Chi. THAT is how needed research for children gets funded. Thanks to them, we have options for treatment as this point. She said when her daughter was sick, they would do radiation on the little spots as they popped up and do some chemo here and there, but there really weren't options. Emmett was a pedal partner for this team 3 years ago and will be again this year! I thought we had a post on the PMC, but apparently we don't because that was a pretty busy time of life. I will put that on my to do list....
While we are excited about the new EZH2 drug, we are a little hesitant because really, who wants their kid to be a guinea pig and work out all the kinks of a new drug? And also, the whole purpose of this particular study is to find out the dosing, which means, how much can they give a kid before the liver and kidney or other organs start having problems. Just in this last week, we have heard that 3 of the kids on the study just dropped out because their tumors grew. Which makes us hesitant. We also found out the adults that did well had solid (body) tumors so the drug could easily get to it. With a brain tumor, a drug has to be able to cross the blood brain barrier, which is made by divine design to be hard to cross, thus protecting your brain. Maybe at a higher dose it will be able to and if we join the study, it will hopefully be a little bit higher of a dose. This is what I mean by working out the kinks. Emmett's quality of life right now is AMAZING and completely asymptomatic so we are drawn to this study because it seems that immediately it will have less impact on his quality of life. The children are closely monitored and the drug is given daily orally at home. But we would still have to be in Boston for 1-2 years (you have to be in clinic to get the drug very regularly and get your check ups and lab draws.... They do offer this study in Denver, so if we choose to go this route, our doctors have agreed that we could work with Denver, too, which is a 6-7 hour drive away. That would be a lot of road travel, but it could be done and would allow us to be home much more without the expense of having to fly to Boston). Right now he does not qualify for this study because his pea size tumor is now half that size thanks to radiation and if it continues to shrink they are not sure it would be from radiation or the new drug, so it wouldn't give accurate study results. More on this later, but he has an MRI this week and if new spots are found on the spine, then he would qualify.
Second option right now is called the Vienna study, and -you guess it-comes from Vienna Austria. It is traditional chemo, and while it is called a study, it is not really, so it could be given at any hospital that is familiar with interthecal chemo, which I don't know if UNMH is, but we would not choose to do chemo at home anyways, so that doesn't matter. Dr. Chi said that although it is chemo, it's not nearly as harsh on the body as the chemo he has been through. However, it is 'labor intensive'/time consuming, in her words. Part of it would be interthecal chemo, which is the one they put through his Rickham Reservoir right into his brain. This is done 5 days a week, every third week and obviously has to be done in clinic. It involves systemic (IV) chemo, interthecal (brain) chemo and radiation, which he has already had. This is about a 1 year protocol also.
The third option, which we haven't considered as much so I don't know as much about is Alisertib, which is St. Jude's research baby right now. We have heard of other families on it with mixed results. Also pretty new and not readily available, but possibly we could get it in Boston, but Dr. Chi would have to apply for compassionate use. I believe it is not 'traditional chemo' (which I define to mean drugs that kill any quickly dividing cells-cancer, hair, throat lining, etc...) but I think it is being used in conjunction with chemo. I guess another option with this is possibly going right to St. Jude in Tennessee for this.
Last option would be to wait and see...? Dr. Chi says they do 'spot-weld' small tumors with radiation sometimes and don't do further treatment. But the haunting question from Dr. MacDonald always remains "But if it has traveled to his brain, where else is it traveling?"
To be honest, Emmett is doing absolutely great still and we are still very slow to call him in recurrence and have clearly not been overly anxious to get treatment started. He will have another MRI this week because the initial MRI on March 3 only checked the radiation spot and not to see if it had spread down his spine or in the old tumor bed. This MRI will be 4 slots instead of 1 like the spine is, so it will definitely be sedated. They said splitting it up into shorter ones without anesthesia makes for choppy pictures and also means he has to have contrast injected into him 4 times instead of 1, which isn't ideal either. We are very hesitant to disrupt his quality of life and are very hopeful he's still fine and will be just fine. He's very bright and excited to be starting kindergarten in the fall and besides some weak muscle tone, really has no side effects from previous treatment. We are hoping not to be pushed urgently into treatment and kind of hope radiation will just do it's thing and maybe do some more treatment just in case, but expecting him to be spot free and clean and life back to normal sometime.
We're grateful for all the prayers and have loved being home some extra time. We still really don't know where we will be in a few weeks, but are starting to expect that by early summer we will be headed back to Boston to discuss and start treatments.
Phew, enough of all that serious chit chat. On to the fun stuff!!
|Team Lick Cancer's Jersey|
|Family Photoshoot right before we left for boston in the fall-we had literally walked out of a doctor appointment and had tons of packing left to do to leave town the next day....but somehow between all the chaos we fit this in and we're glad we did|
|oldie but goodie|
|the whole team in 2013 at the end of the 2 day ride in Provincetown, MA|
|my numbers were way off-looks like they raised almost a half million dollars in one year! That is the complete funding for this EZH2 study. Absolutely amazing! Janet is 2nd from the right and Dr. Chi is holding the check next to her|
|Our Annual Winter Family Campout|
|Matching Easter-they both fit in last year's clothes! Score!|
|Micah graduated from his neat internship|
|Showing off eating skills to his awesome and tough uncle|
|'Honey I shrunk the kids' garden|
|on a bug hunt|
|Flashes of Hope (before we left Boston-photographers volunteer their time and do a free photo session and prints! IN clinic, which is AWESOME because it's one less appointment and they totally accommodate you)|
|visitors at the temple|
|Breakfast in bed...i mean bath|
|Watching General Conference and taking notes|
|friends from az!!|
|top of Sandia Peak|
|'Big brother excavation co' and 'l'il bro wrecking co' how cute is that? My sis is pretty sweet|